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Year : 2008  |  Volume : 3  |  Issue : 2  |  Page : 154-156

Primary intraspinal primitive neuroectodermal tumor: A case report and review of literature

1 Department of Neurosurgery, General Hospital of Tianjin Medical University, Tianjin; Department of Pediatric Neurosurgery, Tianjin Children’s Hospital, Tianjin, China
2 Department of Pediatric Neurosurgery, Tianjin Children's Hospital, Tianjin, China
3 Department of Neurosurgery, General Hospital of Tianjin Medical University, Tianjin, China
4 Department of Pathology, Tianjin Children's Hospital, Tianjin, China

Correspondence Address:
Chang-Hong Shen
Department of Neurosurgery, General Hospital of Tianjin Medical University, Tianjin, 300052
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/1817-1745.43647

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Primary intraspinal primitive neuroectodermal tumors (PNETs) are rare. We report a 3-year-old boy presented with rapidly progressive paraparesis and neurogenic bladder. A thoracolumbar magnetic resonance imaging demonstrated an intrasipnal mass from the T12 to L3 level. An operation was performed with gross total tumor removal. Histologic examination revealed a small round cell tumor and immunohistochemical characteristics of PNET. There was no clinical or radiological evidence for existence of intracranial tumor. The parents denied chemotherapy and radiotherapy. Metastases to lung was noted at 6 months after surgery and died due to respiratory failure at his local hospital. The clinical, imaging, and pathological features are discussed with a review of the literature.

Keywords: Child, magnetic resonance imaging, primitive neuroectodermal tumor, spinal cord

How to cite this article:
Cai CQ, Zhang QJ, Shen CH, Hu XL. Primary intraspinal primitive neuroectodermal tumor: A case report and review of literature. J Pediatr Neurosci 2008;3:154-6

How to cite this URL:
Cai CQ, Zhang QJ, Shen CH, Hu XL. Primary intraspinal primitive neuroectodermal tumor: A case report and review of literature. J Pediatr Neurosci [serial online] 2008 [cited 2023 Dec 3];3:154-6. Available from: https://www.pediatricneurosciences.com/text.asp?2008/3/2/154/43647

   Introduction Top

Primitive neuroectodermal tumors (PNETs), a term proposed in 1973 by Hart and Earle, [1] are a group of malignant neoplasms which derived from the primitive neural crest and mainly exist in the central nervous system, chest wall, lower extremities, trunk, kidney, and orbit, but rarely in the spine. [2],[3],[4],[5] Though multidisciplinary treatments have been well established as the standard therapy for intracranial PNETs, little is known about the optimal treatment strategy and therapeutic results for intraspinal PNETs. The following report illustrates a primary intraspinal PNET at the level of T12 to L3 in a young boy.

   Case Report Top

A 3-year-old boy presented with rapidly progressive paraparesis of lower extremities for 1 month and urinary incontinence for 3 days was admitted to Tianjin Children's Hospital by his parents. He had been able to walk by herself at 11 months old. He had been delivered normally and was healthy otherwise. There was no history of trauma. On admission, he was inambulatory and complications included dysfunction of urination and defecation. Physical examination showed positive tenderness and percussive pain in the lumbar spine and pain hyposensitivity in the lower extremities; the muscle power of 2/5 in right leg and 0/5 in left leg; both ankle and knee jerk reflexes were reduced. Anal reflex was absent. The curvature of the spine was normal. Preoperative magnetic resonance imaging (MRI) scan of the thoracolumbar spine clearly demonstrated a 6 x 1.5 x 1 cm 3 intrasipnal mass from the T12 to L3 level. The signal of the tumor was higher than muscle and similar to vertebrae on the T 1 -weight, but the tumor displayed high signal on the T 2 -weight image [Figure 1A],[Figure 1B]. The edge of lesion strong enhanced on the administration of contrast agent [Figure 2A],[Figure 2B]. Adjacent vertebraes and the disks had a normal signal. MRI scans of the whole spine and head did not find any abnormality. With the clinical impression of intraspinal tumor, the patient was operated on under general anesthesia and a posterior laminectomy of T12 to L3 was performed. Upon examination the tumor was soft and reddish-gray, extending from the level of T12 to the level of L3, approximate 5.5 x 1.5 x 1 cm 3 in size; it grew on left-posterior sides of the spinal cord, and the dura mater was significantly compressed; fortunately, no lesion penetrated into the dura mater. Complete resection of the lesion was achieved and then the spinal cord was pulsated. We did not use intraoperative monitoring. The process of operation was uneventful. The tissue removed at surgery was fixed in 10% buffered formalin. Sections were routinely processed and embedded in paraffin, 5-µm sections were cut and stained with hematoxylin and eosin (H and E). The histopathology of the lesion showed the tumor cells were mainly small and round morphologically, arrayed in slot, with hyperchromatic nuclei, and a small cytoplasmatic wall under a light microscope. Some Homer-Wright rosettes and focal areas of necrosis were found (H and E, x100) [Figure 3A]. Immunohistochemistry showed CD99 (+) [Figure 3B], neuron-specific enolase (NSE) (+), and leucocyte common antigen (LCA) (-). According to clinical and pathological findings, the diagnosis of PNET was made. Postoperatively, the patient's motor weakness of the lower extremities were improved to the level of grade 3/5 in the right and 2/5 in the left. And urinary incontinence was no change. Response to pain was recovery. No other therapies (chemotherapy and/or radiotherapy) were performed according to the parents' opinion. Six months later, the boy was admitted to his local hospital for difficult respiration. Chest X-ray showed metastasis to lung. The parents refused treatment any more and the boy died due to respiratory failure at the local hospital. An autopsy was not permission.

   Discussion Top

The concept of PNETs has been controversial for more than a decade. In the recently updated World Health Organization Classification, PNET is defined as an embryonal tumor composed of undifferentiated or poorly differentiated neuroepithelial cells which have the capacity for or display divergent differentiation along neuronal astrocytic, ependymal, muscular, or melanotic lines. Peripheral primitive neuroectodermal tumors (pPNETs) and Ewing's sarcoma (ES) are closely related malignant, small, and round-cell tumors of soft tissue and bones. Both pPNETs and ES strongly express the glycoprotein p30/32 (CD99), which is encoded by the microneme protein 2 (MIC2) gene. Because of immunohistochemical, ultrastructural and molecular biologic similarities, pPNETs and ES have recently been categorized into the Ewing family of tumors. Despite histologic resemblance, a classification scheme has been proposed for the differential diagnosis of pPNETs and ES based on the recognition of neural differentiation characterized histologically by the presence of Homer-Wright rosettes and/or immunohistochemically. [5],[6],[7]

The most frequent sites of PNETs are the intracranial, chest wall, lower extremities, trunk, kidney, and orbit; however, these tumors rarely originate from the spine. [2],[3],[4],[5] Most cases of PNETs in volving the spinal cord are "drop" metastases from primary intracranial tumors. [8] Our patient underwent repeated imaging of head without a sign for a cerebral primary tumor. No matter if PNET originated from spinal cord, or from vertebrae or from soft tissue, these PNETs often present similar symptoms, therapy, and complications because the spinal cord and spinal nerve roots were in the region. In this case, the tumor was extradural and occupied the spinal canal without penetrating into the dural theca, which suggested that the tumor was not derived from spinal cord and should be classified as pPNET. Based on Lantos et al , [9] report, radiotherapy aids in better control of local recurrence, and chemotherapy may reduce the risk for distant matastases. Despite being gross total resected tumor, the patients died 6 months after surgery from lung metastasis. The results of this case suggest that PNETs in the spinal region may be highly malignant. Chemotherapy and/or radiotherapy should be given to the patients.

No more than 30 cases with primary intraspinal PNETs were previously reported. [5],[8],[10],[11],[12] The age range from 3 months to 69 years, and 16 were younger than 18 years old. Spinal PNET may arise from all levels of the spine and can be intramedullary, extramedullary/intradural, or extradural. The intramedullary tumors might originate from spinal cord, while the extradural tumors might arise from vertebrae, soft tissue, or spinal nerve roots, which possibly belonged to pPNETs. Most of these tumors were impossible to resect en bloc, because it usually involved nerve roots, spinal cord, and vertebrae. Nineteen patients got radiotherapy with the dose of radiotherapy from 30.6 to 56 Gy, but the optimal radiation dose for each patient is difficult to define. Radiotherapy is an important adjuvant therapy for those radiosensitive tumors, particularly in patients with incompletely resected primary tumor. But radiotherapy is associated with a higher incidence of intellectual impairment, endocrine disturbances and growth retardation in young children. Fifteen patients were given chemotherapy, and the most commonly used chemotherapeutic agents were vincristine, adriamycin, cyclophosphamide, ifosfamide, and actinomycin-D. Chemotherapy is the only treatment in children <2 years old, because of severe side effects of irradiation in this age group. Peripheral blood stem cell transplantation was also employed to some PNET patients, but the clinical outcome still needs to be evaluated in a prospective trial. [13],[14],[15],[16] The prognosis of PNET in the spine was very poor and most patients had recurrence/metastasis. The average time from the operation to death was 18.1 months.

In conclusion, PNET originating from the spine is very rare and extremely malignant. The prognosis of these tumors is very poor and its treatment is far from satisfactory. Surgical resection combined with radiation therapy and multidrug chemotherapy are the preferred treatment for these patients.

   References Top

1.Hart MN, Earle KM. Primitive neuroectodermal tumors of the brain in children. Cancer 1973;32:890-7.  Back to cited text no. 1  [PUBMED]  
2.Kushner BH, Hajdu SI, Gulati SC. Extracranial primitive neuroectodermal tumors: The Memorial Sloan-Kettering Cancer Center experience. Cancer 1991;67:1825-9.  Back to cited text no. 2    
3.Pomara G, Cappello F, Cuttano MG. Primitive neuroectodermal tumor (PNET) of the kidney: A case report. BMC Cancer 2004;26:3.  Back to cited text no. 3    
4.Sen S, Kashyap S, Thanikachalam S. Primary primitive neuroectodermal tumor of the orbit. J Pediatr Ophthalmol Strabismus 2002;39:242-4.  Back to cited text no. 4    
5.Kumar R, Reddy SJ, Wani AA, Pal L. Primary spinal primitive neuroectodermal tumor: Case series and review of the literature. Pediatr Neurosurg 2007;43:1-6.   Back to cited text no. 5  [PUBMED]  [FULLTEXT]
6.Kleihues P, Louis DN, Scheithauer BW. The WHO classification of tumors of the nervous system. J Neuropathol Exp Neurol 2002;61:215-25.  Back to cited text no. 6    
7.Mawrin C, Synowitz HJ, Kirches E, Kutz E, Dietzmann K, Weis S. Primary primitive neuroectodermal tumor of the spinal cord: Case report and review of the literature. Clin Neurol Neurosurg 2002;104:36-40.   Back to cited text no. 7  [PUBMED]  [FULLTEXT]
8.Albrecht CF, Weiss E, Schulz-Schaeffer WJ, Albrecht T, Fauser S, Wickboldt J, et al . Primary intraspinal primitive neuroectodermal tumor: Report of two cases and review of the literature. J Neurooncol 2003;61:113-20.   Back to cited text no. 8  [PUBMED]  [FULLTEXT]
9.Lantos PL, Vandenberg SR, Kleihues P. Tumours of the nervous system. In: Graham DI, Lantos PL, editors. Greenfield's neuropathology, 6 th ed., vol. 2, London: Arnold. 1997. p. 583-879.   Back to cited text no. 9    
10.Kampman WA, Kros JM, De Jong TH, Lequin MH. Primitive neuroectodermal tumours (PNETs) located in the spinal canal; the relevance of classification as central or peripheral PNET: Case report of a primary spinal PNET occurrence with a critical literature review. J Neurooncol 2006;77:65-72.  Back to cited text no. 10  [PUBMED]  [FULLTEXT]
11.Deme S, Ang LC, Skaf G, Rowed DW. Primary intramedullary primitive neuroectodermal tumor of the spinal cord: Case report and review of the literature. Neurosurgery 1997;41:1417-20.   Back to cited text no. 11  [PUBMED]  [FULLTEXT]
12.Isolato PA, Agbi C, Davidson B. Primary primitive neuroectodermal tumor of the cauda equina. Hum Pathol 2000;31:999-1001.  Back to cited text no. 12    
13.Harimaya K, Oda Y, Matsuda S. Primitive Neuroecto dermal tumorand extraskeletal Ewing sarcoma arising primarily around the spinal column: Report of four cases and a review of the literature. Spine 2003;28:E408-12.  Back to cited text no. 13    
14.Snyder KM, Mackall CL. Therapy for metastatic ESFT: Is it time to ask new questions? Pediatr Blood Cancer 2007;49:115-6.  Back to cited text no. 14  [PUBMED]  [FULLTEXT]
15.Burke MJ, Walterhouse DO, Jacobsohn DA, Duerst RE, Kletzel M. Tandem high-dose chemotherapy with autologous peripheral hematopoietic progenitor cell rescue as consolidation therapy for patients with high-risk Ewing family tumors. Pediatr Blood Cancer 2007;49:196-8.  Back to cited text no. 15  [PUBMED]  [FULLTEXT]
16.Pradhan KR, Johnson CS, Vik TA, Sender LS, Kreissman SG. A novel intensive induction therapy for high-risk neuroblastoma utilizing sequential peripheral blood stem cell collection and infusion as hematopoietic support. Pediatr Blood Cancer 2006;46:793-802.  Back to cited text no. 16  [PUBMED]  [FULLTEXT]


  [Figure 1A], [Figure 1B], [Figure 2A], [Figure 2B], [Figure 3A], [Figure 3B]

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